Chapter 11 Kawasaki Disease

Kawasaki disease (KD) is an acute febrile systemic vasculitis usually occurring in children younger than five years, and rarely reported in neonates and adults. This chapter discusses the epidemiology, etiology, pathogenesis, clinical manifestations, and treatments of KD. The etiology still remains unknown, although epidemiological and clinical features strongly suggest an infectious cause. Immunological abnormalities in the acute phase of the disease reflect activation of immune system and marked production of cytokines by activated cells. KD has some similarities to toxin-mediated diseases, both from a clinical and an immunological point of view. The role of one or more superantigens competent of stimulating large numbers of T cells produced by certain strains of Staphylococcus or Streptococcus is discussed in the chapter, in the context of the etiology of KD. Atypical cases are those with fever, acute surgical symptoms, or neurological manifestations as presenting signs. Medical history, physical examination, and laboratory tests including elevated white-blood cell (WBC) count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and low hemoglobin, sodium and albumin levels may help to rule out illnesses mimicking KD. Oral or pulsed corticosteroids in children refractory to intravenous immunoglobulins (IVIG) are an alternative and safe treatment.